Victoria P. Werth, Dédée F. Murrell, Pascal Joly, Renata Heck, Jamie M. Orengo, Marius Ardeleanu, Verena Hultsch
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引用次数: 0
摘要
大疱性类天疱疮（BP）是一种自身免疫性水疱病，多发于老年人，对生活质量有很大的负面影响。该病的主要特征是半色素体蛋白 BP180 和/或 BP230 的自身抗体，以及具有显著 2 型炎症特征的炎症反应，包括血清 IgE 升高、皮损和外周血中嗜酸性粒细胞数量增加，以及皮损中 2 型细胞因子和趋化因子表达升高。在这篇综述中，我们介绍了目前已知的 BP 病理生理学，包括 2 型炎症的作用，并讨论了针对 2 型免疫介质的生物制剂的研究结果如何有助于阐明驱动 BP 病理生理学的生物机制。未来对这些靶向疗法和其他正在开发的疗法的研究将有助于进一步阐明血压病理生理学的基础机制，并有可能为患者提供更好的治疗方案。
Pathophysiology of Bullous Pemphigoid: Role of Type 2 Inflammation and Emerging Treatment Strategies (Narrative Review)
Bullous pemphigoid (BP) is an autoimmune blistering disease that most often affects elderly individuals and has a significant negative impact on quality of life. The disease is characterized primarily by autoantibodies to hemidesmosomal proteins BP180 and/or BP230, and an inflammatory reaction with notable features of type 2 inflammation, including elevated serum IgE, increased numbers of eosinophils in lesions and peripheral blood, and elevated expression of type 2 cytokines and chemokines in skin lesions. In this review, we present what is known about BP pathophysiology, including the role of type 2 inflammation, and discuss how findings from studies of biologics targeting type 2 immune mediators have helped to clarify the biological mechanisms driving BP pathophysiology. Future studies of these targeted therapies and others in development will help to further elucidate the mechanisms underlying BP pathophysiology and potentially provide better treatment options for patients.
期刊介绍：
Advances in Therapy is an international, peer reviewed, rapid-publication (peer review in 2 weeks, published 3–4 weeks from acceptance) journal dedicated to the publication of high-quality clinical (all phases), observational, real-world, and health outcomes research around the discovery, development, and use of therapeutics and interventions (including devices) across all therapeutic areas. Studies relating to diagnostics and diagnosis, pharmacoeconomics, public health, epidemiology, quality of life, and patient care, management, and education are also encouraged.
The journal is of interest to a broad audience of healthcare professionals and publishes original research, reviews, communications and letters. The journal is read by a global audience and receives submissions from all over the world. Advances in Therapy will consider all scientifically sound research be it positive, confirmatory or negative data. Submissions are welcomed whether they relate to an international and/or a country-specific audience, something that is crucially important when researchers are trying to target more specific patient populations. This inclusive approach allows the journal to assist in the dissemination of all scientifically and ethically sound research.
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